Abstract
Summary A case of incontinentia pigmenti in a 3-week-old girl, with a 3-month follow-up examination, has been presented. The child showed the classic dermatologic, ocular, and dental involvement. The question of etiology is still under investigation, and evidence is developing that may discount genetics as the causative factor. A plasma protein deficiency, although not generally reported in the literature, may be the etiologic factor. Dental pathosis included oligodontia with atypical aberrations of the crown. The eruption sequence of the teeth will remain a question until the patient is older. The mentality of the child will also remain an unknown factor during her early development. Close observation through tests and radiographic examination will be an important consideration in this child's development.
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